Abstract:
:Variants of isovaleryl-CoA dehydrogenase (IVDHase, EC 1.3.99.10) in 15 isovaleric acidemia fibroblast lines were analyzed using [35S]methionine labeling, immunoprecipitation with anti-rat IVDHase antiserum, and NaDodSo4/polyacrylamide gel electrophoresis. Five distinct variants of IVDHase were detected. The molecular size of variant 1 (43 kDa) was indistinguishable from that of normal IVDHase (43 kDa), although the activity of this enzyme was as deficient (0-2.2% of normal control) as that of any other variant. It was synthesized as a precursor (45 kDa), which is the case for normal IVDHase. Variant 2 was synthesized as a 42-kDa precursor, but only a small portion of it was processed to the mature variant form (40 kDa). Variant 3 (41 kDa) was synthesized as a 43-kDa precursor. Variant 4 (40 kDa) was synthesized as a 42-kDa precursor that was readily processed to the mature form. In cells with variant 5, no material that crossreacted with the anti-rat IVDHase antibody was detected. These results suggest that variant 1 may be due to a point mutation, while variants 2-4 may be encoded by a different mutant IVDHase allele that causes the premature termination of translation, although other complex mechanisms are possible. A deletion, a nonsense mutation close to the NH2 terminus or an extremely labile mRNA may give rise to variant 5.
journal_name
Proc Natl Acad Sci U S Aauthors
Ikeda Y,Keese SM,Tanaka Kdoi
10.1073/pnas.82.20.7081subject
Has Abstractpub_date
1985-10-01 00:00:00pages
7081-5issue
20eissn
0027-8424issn
1091-6490journal_volume
82pub_type
杂志文章abstract::Conformational aspects of the acetylcholine receptor (AcChoR) of Electrophorus electricus have been examined by studies of its interaction with structurally related, constrained aromatic bis quaternary compounds. Among the compounds synthesized was 3,3'-bis[alpha-(trimethylammonium)-methyl]azobenzene dibromide (3,3'-b...
journal_title:Proceedings of the National Academy of Sciences of the United States of America
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doi:10.1073/pnas.76.1.256
更新日期:1979-01-01 00:00:00
abstract::Protein kinase C (PKC) phosphorylation stimulates the cystic fibrosis transmembrane conductance regulator (CFTR) channel and enhances its activation by protein kinase A (PKA) through mechanisms that remain poorly understood. We have examined the effects of mutating consensus sequences for PKC phosphorylation and repor...
journal_title:Proceedings of the National Academy of Sciences of the United States of America
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doi:10.1073/pnas.0303411101
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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更新日期:2011-06-14 00:00:00
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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abstract::Integrated hepatitis B virus (HBV) DNA is almost invariably found in hepatocellular carcinomas (HCC) which develop in HBV carriers. Integrated HBV DNAs from two single-integration HCCs (C3 and C4) have been cloned, and the cellular integration sites have been analyzed. Integrated HBV DNA of C3 is present in chromosome...
journal_title:Proceedings of the National Academy of Sciences of the United States of America
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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doi:10.1073/pnas.82.3.668
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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abstract::The high-affinity (K(m) = 3 x 10(-8) M) transport system for histidine in Salmonella typhimurium has been resolved into three components: J, K, and P. J, which is a histidine-binding protein released by osmotic shock, is specified by the hisJ gene: hisJ mutants lack the binding protein and are defective in histidine t...
journal_title:Proceedings of the National Academy of Sciences of the United States of America
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更新日期:1970-08-01 00:00:00
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更新日期:1993-08-01 00:00:00
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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doi:10.1073/pnas.0701967104
更新日期:2007-06-26 00:00:00
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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doi:10.1073/pnas.75.10.5006
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
pub_type: 杂志文章
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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doi:10.1073/pnas.86.8.2693
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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更新日期:1988-09-01 00:00:00
abstract::The role of microtubules in 3H-labeled protein secretion in rat lacrimal glands was probed by the use of colchicine and two of its analogues that reversibly bind to tubulin. These analogues were 2-methoxy-5-(2,3,4,4'-trimethoxyphenyl)-2,4,6-cycloheptatriene-1-o ne and 2,3,4,4'-tetramethoxy-1,1'-biphenyl, the latter ha...
journal_title:Proceedings of the National Academy of Sciences of the United States of America
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更新日期:1989-06-01 00:00:00
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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更新日期:2006-12-12 00:00:00
abstract::Forkhead box g1 (Foxg1) is a nuclear-cytosolic transcription factor essential for the forebrain development and involved in neurodevelopmental and cancer pathologies. Despite the importance of this protein, little is known about the modalities by which it exerts such a large number of cellular functions. Here we show ...
journal_title:Proceedings of the National Academy of Sciences of the United States of America
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abstract::The crystal structure of the murine Fab S-20-4 from a protective anti-cholera Ab specific for the lipopolysaccharide Ag of the Ogawa serotype has been determined in its unliganded form and in complex with synthetic fragments of the Ogawa O-specific polysaccharide (O-SP). The upstream terminal O-SP monosaccharide is sh...
journal_title:Proceedings of the National Academy of Sciences of the United States of America
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更新日期:2000-07-18 00:00:00
abstract::Optimization theory has been used to analyze evolutionary adaptation. This theory has explained many features of biological systems, from the genetic code to animal behavior. However, these systems show important deviations from optimality. Typically, these deviations are large in some particular components of the sys...
journal_title:Proceedings of the National Academy of Sciences of the United States of America
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更新日期:2009-12-01 00:00:00
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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doi:10.1073/pnas.1601777113
更新日期:2016-06-21 00:00:00
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journal_title:Proceedings of the National Academy of Sciences of the United States of America
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doi:10.1073/pnas.90.5.1652
更新日期:1993-03-01 00:00:00
abstract::We have identified a cDNA from a human phytohemagglutinin-activated lymphoblast library encoding a protein that binds 125I-labeled human interleukin 12 (125I-huIL-12) with a Kd of about 5 nM when expressed in COS-7 cells. When coexpressed in COS-7 cells with the previously identified IL-12 beta receptor (IL-12R beta) ...
journal_title:Proceedings of the National Academy of Sciences of the United States of America
pub_type: 杂志文章
doi:10.1073/pnas.93.24.14002
更新日期:1996-11-26 00:00:00